Pulmonary Arterial Hypertension (PAH)


Pulmonary Arterial Hypertension (PAH) is the medical term for a progressive disease caused by narrowing or tightening (constriction) of the pulmonary arteries that connect the right side of the heart to the lungs. PAH is a serious condition that affects the blood vessels (pulmonary arteries) that carry blood from the heart to the lungs.

Although Pulmonary Arterial Hypertension is an uncommon condition, it is a disease that can affect males and females of all ages and ethnic backgrounds. Very rarely, PAH occurs in childhood.

PAH gradually restricts the flow of blood through the lungs. As a result, the pressure in the pulmonary arteries increases. The heart must pump against this increased pressure to maintain blood flow in the lungs and to the rest of the body. Over time, this can affect the heart’s ability to work effectively and may eventually lead to heart failure.

There are many causes of PAH and your doctor will perform several tests to confirm that you have PAH and to look for a cause. Causes of PAH may include:

  • Idiopathic (meaning the cause is unknown) PAH
  • Heritable (genetic causes that may be passed to your children) Drug and toxin induced

Associated with other systemic diseases such as:

  • Connective tissue disease such as scleroderma, lupus, rheumatoid arthritis
  • HIV infection
  • Congenital heart disease
  • Liver disease

Some people may develop PAH if there is a family history (heritable) of the disease, while others develop PAH for no known reason (called idiopathic PAH). Because it can be genetically inherited in some cases, once the diagnosis is made it is important for other close relatives to be screened for PAH. You can receive guidance from your expert treatment centre regarding this.

  • The first symptoms of Pulmonary Arterial Hypertension may be very slight or difficult to notice, and you may only notice them when you exercise – climbing stairs, running, walking uphill or carrying bags. However, as PAH progresses, the symptoms may become more noticeable with less activity and interfere with daily routine.

    Common Pulmonary Arterial Hypertension Symptoms include:

  • Shortness of breath – breathlessness, especially with physical activity
  • Feeling tired or fatigued
  • Dizziness
  • Chest discomfort or pain
  • A fast and/or irregular heartbeat (also called palpitations) Light-headedness or fainting
  • Swelling in your legs, and /or the belly (also called fluid retention or oedema) Lips and fingers turning purple or blue

Many of these symptoms are nonspecific and can be due to other diseases that affect the heart and lungs. For this reason it is important that the diagnosis of PAH is made by a physician who is an expert in this area.

An accurate diagnosis of Pulmonary Arterial Hypertension is important, so that you can get the correct treatment. It is often difficult to recognise PAH because breathlessness and fatigue are common symptoms that are often overlooked or thought to be caused by many of the other diseases that affect the heart and lungs.

A PAH diagnosis requires a heart catheterisation and many other tests to exclude left heart disease, lung disease and other rare disorders. The diagnosis of a patient with PAH can be investigated by:

  • Chest X-ray
  • ECG (Recording of the heart’s electrical activity using patches on your skin) Various blood tests
  • Breathing tests (Lung function tests) Ultrasound of the heart (Echocardiogram)
  • Scan to compare blood and air circulation in the lungs (V/Q scan) CT scans (Looks in depth at the tissue in the heart and lungs)

A right heart catheterisation (RHC) is typically undertaken to confirm the diagnosis of PAH.

Pulmonary Arterial Hypertension is a severe condition and there is no cure at the present time. However, many PAH treatments are now available that can improve your symptoms, quality of life and keep the condition under control. Everyone’s disease is different and taking your treatment as prescribed can help keep you from getting worse. Early treatment is important since PAH can be a progressive disease.

PAH treatment options will depend on the cause of PAH and the stage at which it is diagnosed. This is not a disease that you should ‘wait and see’ to do something about. It usually gets worse with time, and we know from studies that the sooner we treat it, the better chance you have of improving.

In the past, doctors had little to offer in terms of treatment options. However, this has changed dramatically over the last 10 years with the introduction of several new medications and treatments. Although currently there is no cure for this disease, it can often be well managed for many years with medications.

Types of PAH Treatment

In general, medical treatment of your Pulmonary Arterial Hypertension is divided into PAH medications or medicines and supportive treatment. PAH medicines are given to dilate or open up the blood vessels of the lungs, improve blood flow to the lungs, and reduce the strain on the heart. Your specialist will discuss with you the different PAH medications and those that may be suitable for you.

Pulmonary Arterial Hypertension Medications

There are several medicines that treat PAH specifically. These medications are most commonly taken as tablets by mouth (orally). Other ways include via a needle into the vein (intravenously) or under the skin (subcutaneously) as well as by inhalation (mist). Each medication to treat PAH works in a distinct way and most patients now are given two or more PAH medications for best results.

Transplant Surgery

Most individuals with PAH do very well with treatments but occasionally and only if these treatments are ineffective or are unable to control the condition, more involved treatments including organ transplant may need to be considered. This is where an organ in your body is replaced with an organ from a donor. Transplant surgery for PAH can involve replacing the heart and lungs, or just the lungs. Transplantation is required less commonly these days because of medications being more effective.

You can contact the Lung Foundation Information and Support service to obtain resources about PAH and be connected to patient support services.

We have recently developed a new resource- Better Living with Pulmonary Arterial Hypertension (PAH) A Patient Guide. This Guide will help you understand more about this disease, the symptoms, treatment options and practical steps that can be taken to live better with the condition.

Patient Support Groups

There are two patient support organisations in Australia for people with Pulmonary Hypertension. These organisations are run by people who have an understanding of Pulmonary Hypertension from a personal perspective.

To receive your copy of the PAH Patient Guide or find out what support services are available in your area please contact our Information and Support Centre on 1800 654 301 or email enquiries@lungfoundation.com.au

You can download the Patient Guide here:

LFA Better Living with PAH A5_0417-WEB (9 MB)

You can download the PAH fact sheet here:

Pulmonary Arterial Hypertension Fact Sheet (136 KB)

International Organisations

 

Lung Foundation Australia acknowledges our foundation sponsor Actelion and supporting sponsor GlaxoSmithKline for their support of our Rare Lung Disease -Pulmonary Arterial Hypertension Program.

 A special thanks to Actelion for their support in developing a new patient resource; Better Living with Pulmonary Arterial Hypertension – A Patient Guide.

Kevin’s story

My name is Kevin, I am 47 years old and a father of three beautiful daughters. In 2010, I was diagnosed with severe Pulmonary Hypertension (PH).

Even before 2010 my body was telling me something wasn’t right but I wasn’t really listening to it. I would get short of breath a lot easier than before and I joked about getting older and unfit.

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Tegan’s story

At the age of 5, my mum was told I had 5 years to live. I have Pulmonary Arterial Hypertension (PAH), which is a progressive and life limiting disease. Access to a trial medication is what has kept me alive and allowed me to live a somewhat normal life. Unfortunately my story is the exception rather than the rule.

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Bridget’s story

My name is Bridget Doyle, I am 21 years old and I live in Brisbane, Queensland with my mum, dad and my dog, Hope. I have two lung conditions Kartagener Syndrome and Pulmonary Arterial Hypertension (PAH).

Read more